Hematology Flashcards
1
Q
brain tumors
A
- age <15, 60% infratentorial, pilocytic astrocytome (MC overall; benign, cystic, slow growing, 6.5-9y, surgical resection), medulloblastoma (MC< malignant, XRT/chemo, resection)
- infant sxs: large head, bulging fontanelle, anorexia/FTT
- child sxs: morning headaches, V (no N), drowsiness, dec academics, personality change
- other sxs: vision change/papilledema, altered speech and handwriting, CN deficit, head tilt, altered gait and ataxia, diabetes insipidus
- dx: neuro exam, head CT or MRI head/spine, bx
- tx: surgery is mainstay
2
Q
lead poisoning
A
- RF: inner city low income, recent immigrants, exposure, adult workers employed in lead exposure jobs, ayurvedic meds
- sxs: asx, pica, anorexia, colicky abdominal pain
- signs: lead lines in gingiva, FTT, mental delay, CNS sxs
- dx: lead (if + get venous CBC), AAP recommends checking at 9-12m and 24m, questionaire (annual 3-6y), CBC (basophilic stippling, hypochromic anemia), XR (lead lines on x-rays of knee/wrist)
- tx: blood levels 10-15 = low IQ, impaired development, hearing and growth; blood levels 25-55 = more severe, renal, hematopoietic damage - IM/IV EDTA; blood levels 45-69 = IM EDTA + PO DMSA; blood levels >70 = IV EDTA + IM DMSA
3
Q
aplastic anemia
A
- maybe autoimmune, 50% idiopathic, radiation, infeciton, toxins, drugs, inherited . = fanconi syndrome
- sxs: weakness, fatigue, palps, DOE, tinnitus, bleeding and bruising, infxns
- dx: CBC (normocytic anemia, neutropenia, thrombocytopenia), peripheral smear shows peripheral pancytopenia with marrow hypocellularity, bone marrow bx to r/o BDS
- tx: transfusion dependent with growth factors, immunosuppression (ATG stops Tcell response, cyclosporin), cure is allogenic stem cell transplant
4
Q
iron deficiency anemia
A
- significant def in mass of circ RBCs (O2 carrying capacity = low
- dec cell mass or Hgb conc; microcytic (MCV <80), hypochromic w/ low H/H
- sxs: fatigue, palps, SOB, weakness, HA, tinnitus
- signs: tachycardia, tachypnea on exert, pallor, glossitis, angular chelitis, pica, koilonychia, jaundice and splenomegaly
- dx: CBC (retic low, RDW = high), dec iron, ferritin, and transferrin, inc TIBC, ferritin <15 (diagnostic), check hgb and hct, periph smear → poikilocytes (pencil or cigar shaped)
- tx: oral iron TID (6wk to correct anemia, 6mo to replete iron) → Ferrous sulfate 3mg/kg once or twice daily, give between meals with juice, not milk
5
Q
sickle cell anemia
A
- mutation in B-globin gene that changes the 6th amino acid form glutamic acid to valine
- sxs: acute pain (hrs to 2wk); RF → infxn, fever, excess exercise, anxiety, abrupt change in temp, hypoxia, hypertonic dyes
- signs: TTP, fever, tachycardia, anxiety
- complications: pulm HTN, ESRD, hand foot syndrome, priapism (permanent impotence)
- dx: hemolytic anemia, reticulocytosis, granulocytosis, periph smear → elongated and crescent RBCs, target cells, nucleated RBC, Hgb electro, mass spec, sickling tests (confirms)
- tx: crisis → vigorous hydration, aggressive pain meds (morphine), nasal O2; severe sxs → hydroxyurea, pts 3+ crises/year or repeated ACS → increases production of Hgb F (cant sickle), BMT
6
Q
B thalessemia
A
- only 2 Bglobin genes on chrom 11, but 4 alpha genes on chrom 16 → disrupts ratio between alpha and beta chains → changes stability of Hb and causes hemolysis
- sxs: asx, mild anemia
- signs: HSM, jaundice
- dx: Hgb electrophoresis, CBC (microcytic hypochromic anemia), iron NL to increased (serum iron, ferritin, transferrin sat, normal TIBC), peripheral smear → target cells, basophilic stippling, elliptocytes
- tx: transfusion, PO iron repletion and B12 only if concomittant iron def anemia, allogenic bone marrow transp, folic acid, deferoxamine (iron chelator), splenectomy
7
Q
megaloblastic anemia
A
- MCV >100, inhib of DNA synth during RBC produciton
- dx: CBC, iron studies (NL to inc serum iron, ferritin, transferrin sat; NL to DEC TIBC), periph smear shows macrocytic megaloblastic cells
8
Q
vit B12 deficiency
A
- vit B12 = cobalamin
- autoimmune destruciton of gastric parietal cells → atrophic gastritis → lack of intrinsic factor produciton; cofactor for 2 enzymatic rxns required for DNA synth, brain/nervous system fn, formation of RBC
- RF: chronic alc, vegetarian, celiac and crohn dz, gastric bypass surg, parasites
- MCC: pernicious anemia (lack of IF)
- sxs: anemia, sore tongue, periph neruopathy, balance probs, depression, dementia, glossitis
- signs: loss of vib touch
- dx: B12 dec, homocysteine inc, methylmalonic acid inc, hyperseg neutrophils
- tx: lifelong IM B12 → cyanocobalamin daily x1wk, weekly x1m, monthly for life
9
Q
folic acid deficiency
A
- cofactor for DNA synth, alcs and malnourished have smaller stores, dec intake, inc requirement, sickle cell, thalassemia, sprue, crohn, drugs
- sxs: less neuro sxs, neural tube defects (spina bifida)
- dx: homocysteine inc, serum folic acid low, RBC folic acid <150 (dxic), macroovalocytes and hyperseg PMNs (pathognomonic)
- tx: po folic acid 1-5mg, avoid ETOH, green leafy veggies, yeast, legumes, fruits, animal proteins, prophylactic folic acid for preg/lactating and sickle cell
10
Q
hemolytic anemias
A
- sxs of acute/chronic anemia, dark urine, back pain
- signs: jaundice
- dx: CBC (high retic), inc iron, ferritin, transferrin sat, dec TIBC, total (indirect) bili inc, haptoglobin dec, periph smear → microcytic, normochromic anemia, spherocytes, bone marrow bx
11
Q
glucose-6 phosphate dehydrogenase deficiency
A
- oxidant sensitive hemolytic dz (heinz bodies + bite cells → RBC destruction)
- MC seen in tropical areas prevalent for malaria (Africa, China, Mediterranean)
- hx: hemolysis only with infxn, met acidosis, and certain meds, chronic hemolytic anemia, jaundice, sx of hemolysis
- dx: periph smear: bite cells, heinz bodies
- tx: avoid potentially harmful drugs, monitor infxn, acute → blood transfusion
12
Q
hereditary spherocytosis
A
- congenital hemolytic jaundice, familial hemolytic anemia
- inherited dysfn or deficiency in one of the erythrocyte membrane proteins (spectrin, ankyrin, band 3 protein, or protein 4.2) → spherocytic erythrocytes are sequestered and destroyed in spleen; autosomal dom
- sxs: mild jaundice, mild-mod anemia, malaise, abd pain in LUQ
- signs: splenomeg
- dx: CBC, MCV dec; retic count, LDH, indirect bili, stool urobili → elevated; hgb low, EDW elevated, spherocytes on periph smear, osmotic fragility inc, coombs test neg
- tx: splenectomy, cholecystectomy if gallstones present
- complications: hypoplastic crises (follow acute viral illness → profound anemia, HA, N, abd pain, pancytopenia, hypoactive marrow, pigmented gallstones
13
Q
acute lymphoblastic leukemia
A
- neoplasm of early lymphocyte precursors, MC in young children <15, MC childhood cancer, peak 2-5y
- RF: initial WBC and age at dx, rate of response, cytogenetics
- sxs: anemia, neutropenia (inc risk of bact infxn), thrombocytopenia (abnl mucosal or cutaneous bleeding, splenomegaly, hepatomegaly, LAD, bone and jnt pain, CNS involvement (meningitis, seizures), testicular involvement (ALL), anterior mediastinal mass (T-cell ALL)
- dx: innunofluorescence assay (TdT +), histology shows lymphoblasts, bone marrow bx required for dx and shows myeloblasts
- labs: WBC count variable with large number of blasts (hyperleukocytosis), anemia, thrombocytopenia, granulocytopenia, electrolytes (hyperuricemia, hyperkalemia, hyperphosphatemia
- tx: most responsive to chemo, 75% achieve full remission, relapses occur but respond well, with aggressive tx, survival rates can be up to 15y or longer
- poor prognostic factors = age <2 or >9, WBC >100000 and CNS involvement
14
Q
indolent or low-grade lymphoma
A
- small lymphocytic lymphoma: closely related to CLL, more common in elderly, indolent course, eventually results in wide-spread involvement with dissemination to liver, spleen, bone marrow
- follicular, small, cleaved-cell lymphoma: most common form of NHL, mean age onset 55y, may transform to diffuse, large cell; associated with translocation, indolent course, presents with painless, peripheral LAD, cured with radiotx but only 15% have localized dz, median survival 10y
15
Q
intermediate lymphoma
A
- diffuse, large B-cell lymphoma: predominantly B-cell origin, middle aged and elderly, locally invasive, presents as large, extranodal mass, 85% cure rate with CHOP tx
